caa a22 4500 445888601 CHVBK 20180317145608.0 cr unu---uuuuu 170323e20110101xx s 000 0 eng 10.1007/s00535-010-0323-4 doi (NATIONALLICENCE)springer-10.1007/s00535-010-0323-4 Clinical significance of immunoglobulin G4-associated autoimmune hepatitis [Elektronische Daten] [Takeji Umemura, Yoh Zen, Hideaki Hamano, Satoru Joshita, Tetsuya Ichijo, Kaname Yoshizawa, Kendo Kiyosawa, Masao Ota, Shigeyuki Kawa, Yasuni Nakanuma, Eiji Tanaka] Background: Immunoglobulin (Ig) G4-associated autoimmune hepatitis (AIH) is a recently identified and possibly new disease entity. However, the epidemiology and clinical features of IgG4-associated AIH remain uncertain. The aim of this study was to determine the prevalence and the clinical, serological, and histological characteristics of IgG4-associated AIH. Methods: We examined the clinical features, serum IgG4 concentration, liver biopsy histology, and IgG4-bearing plasma cell infiltration of 60 patients with type 1 AIH and 22 patients with autoimmune pancreatitis. Results: High serum IgG4 concentration (≥135mg/dL) and IgG4-bearing plasma cell infiltration in the liver (≥10/high-power fields [HPFs]) were found in 2 of the 60 (3.3%) patients with type 1 AIH. These patients had high serum levels of IgE, giant cell change, and rosette formation in the liver. Although corticosteroid therapy reduced the serum IgG4 concentration and normalized liver enzymes and histology, one patient developed IgG4-related sclerosing cholangitis after 5years of follow-up. Conclusions: Because IgG4-associated AIH was found in over 3% of Japanese patients with type 1 AIH in our cohort, further studies are needed on this possible new disease entity and its impact on the diagnostic guidelines of AIH. Springer, 2010 Autoimmune hepatitis nationallicence Autoimmune pancreatitis nationallicence IgG4 nationallicence Histology nationallicence Sclerosing cholangitis nationallicence Umemura Takeji Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut Zen Yoh Division of Pathology, Kanazawa University Hospital, Kanazawa, Japan aut Hamano Hideaki Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut Joshita Satoru Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut Ichijo Tetsuya Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut Yoshizawa Kaname Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut Kiyosawa Kendo Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut Ota Masao Department of Legal Medicine, Shinshu University School of Medicine, Matsumoto, Japan aut Kawa Shigeyuki Center for Health, Safety, and Environmental Management, Shinshu University, Matsumoto, Japan aut Nakanuma Yasuni Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan aut Tanaka Eiji Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut Journal of Gastroenterology Springer Japan 46(2011-01-01), 48-55 0944-1174 46<48 2011 46 535 https://doi.org/10.1007/s00535-010-0323-4 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00535-010-0323-4 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Umemura Takeji Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut NATIONALLICENCE 700 1- Zen Yoh Division of Pathology, Kanazawa University Hospital, Kanazawa, Japan aut NATIONALLICENCE 700 1- Hamano Hideaki Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut NATIONALLICENCE 700 1- Joshita Satoru Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut NATIONALLICENCE 700 1- Ichijo Tetsuya Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut NATIONALLICENCE 700 1- Yoshizawa Kaname Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut NATIONALLICENCE 700 1- Kiyosawa Kendo Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut NATIONALLICENCE 700 1- Ota Masao Department of Legal Medicine, Shinshu University School of Medicine, Matsumoto, Japan aut NATIONALLICENCE 700 1- Kawa Shigeyuki Center for Health, Safety, and Environmental Management, Shinshu University, Matsumoto, Japan aut NATIONALLICENCE 700 1- Nakanuma Yasuni Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan aut NATIONALLICENCE 700 1- Tanaka Eiji Division of Hepatology and Gastroenterology, Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621, Matsumoto, Japan aut NATIONALLICENCE 773 0- Journal of Gastroenterology Springer Japan 46(2011-01-01), 48-55 0944-1174 46<48 2011 46 535 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer